In particular, SMARCB1 is involved in ES, but also in malignant rhabdoid tumors (MRT), atypical teratoid/rhabdoid tumors (AT/RT) of the central nervous system, malignant peripheral nerve sheath tumors (MPNST), myoepithelial neoplasms and renal medullary carcinomas (RMC) [19,44,45,46]. The gene discussed is SMARCB1; the disease is epithelioid sarcoma.